Study on the Treatment of Nonthrombotic Obstructive Pulmonary Hypertension

NCT06980584 | Recruiting

• 1 other identifier: 2025-KE-61_Int
• Study Type: interventional
• Target: 48
• Locations: 1 country
• Sites: 1

Brief Summary

Through a randomized controlled trial (RCT) design, this study aiming to evaluated the efficacy and safety of rituximab lymph node injection combined with pulmonary vascular interventional therapy in treating fibrosing mediastinal pulmonary hypertension (FM-PH).Eligible participants were randomly assigned to either the combined treatment group, receiving both pulmonary vascular intervention and rituximab lymph node injection, or the interventional-only group, which received pulmonary vascular intervention alone. At 3, 6, and 12 months post-treatment, the efficacy was assessed based on symptom improvement, hemodynamic changes, lesion volume reduction, etc. Safety was mainly evaluated by comparing adverse event incidence between the two groups.

Conditions

Fibrosing Mediastinitis; Pulmonary Hypertension

Keywords

Rituximab; Endobronchial Ultrasound; Pulmonary Vascular Intervention

Outcome Measures

Primary Outcomes (2)

• Change from Baseline in the Six Minutes Walk Distance at 12 Months.

  Conduct 6-minute walk test to measure the 6-minute walk distance, and compare the walking distances at baseline and 12 months after treatment.

  From enrollment to the end of treatment at 12 months

• The incidence of treatment-related serious adverse events (SAE)

  The incidence of treatment-related serious adverse events is measured by tracking and documenting any SAEs that occur during the treatment period, as well as through follow-up assessments. Each event is evaluated for severity and causality, and the incidence is calculated by dividing the number of patients who experience at least one treatment-related SAE by the total number of treated patients.

  During the one-year follow-up period after treatment completion

Secondary Outcomes (14)

• Change from baseline in the World Health Organization Functional Classification of Pulmonary Hypertension at 3, 6 and 12 months.

  From enrollment to the end of treatment at 3, 6 and 12 months.

• Change from Baseline in the mean pulmonary artery pressure (mPAP) at 3 and 12 Months.

  From enrollment to the end of treatment at 3 and12 months

• Change from Baseline in the Cardiac Index (CI) at 3 and 12 Months.

  From enrollment to the end of treatment at 3 and12 months

• Change from Baseline in the Pulmonary Vascular Resistance (PVR) at 3 and 12 Months.

  From enrollment to the end of treatment at 3 and12 months

• Change from Baseline in the forced vital capacity (FVC) at 3, 6 and 12 Months.

  From enrollment to the end of treatment at 3, 6 and 12 months

Study Arms (2)

Combined treatment group

EXPERIMENTAL

Participants assigned to this group will receive both pulmonary vascular intervention and rituximab lymph node injection.

Procedure: Rituximab Lymph Node Injection Combined with Pulmonary Vascular Intervention

Interventional-only group

ACTIVE COMPARATOR

Participants assigned to this group will receive pulmonary vascular intervention alone.

Procedure: Pulmonary Vascular Interventional Therapy

Interventions

Rituximab Lymph Node Injection Combined with Pulmonary Vascular Intervention PROCEDURE

Based on the degree of vascular stenosis, individualized and periodic pulmonary vascular interventions are performed to reopen obstructed vessels. During the treatment cycle, one lymph node drug injection is administered: 50mg of rituximab is dissolved in 15ml of 5% glucose solution and injected at multiple sites into the identified enlarged mediastinal lymph nodes under ultrasound-guided bronchoscopy.

Combined treatment group

Pulmonary Vascular Interventional Therapy PROCEDURE

According to the degree of vascular stenosis, individualized and periodic pulmonary vascular interventions are performed to reopen obstructed pulmonary vessels.

Interventional-only group

Eligibility Criteria

• Age: 18 Years - 85 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• Diagnosed with fibrosing mediastinitis between November 2024 and November 2026, aged between 18 and 85 years.
• The patient presented with symptoms of chest tightness, shortness of breath, and reduced exercise tolerance.
• Chest CT revealed mediastinal lymph node compression of the pulmonary artery, with evidence of pulmonary hypertension consistent with the patient's symptoms.
• The subject signed the informed consent form prior to participation and is able to comply with the study protocol and one-year follow-up.

You may not qualify if:

• Currently in the active phase of infection, including but not limited to tuberculosis, Histoplasma capsulatum, and Aspergillus infections;
• The underlying primary disease, such as sarcoidosis, Behcet's disease, or uncontrolled IgG4-related disease, is currently not well controlled.
• Before treatment, a large amount of pleural effusion was still present.
• Pulmonary function tests (PFT) showed FEV1 \<30% of the predicted value, FEV1/FVC \<30%, and DLCO \<30%.
• There are contraindications to bronchoscopy or endovascular intervention.
• Complicated by other end-stage organ dysfunction, such as Child-Pugh class C liver function or stage IV chronic renal failure.
• Complicated by severe immunosuppression.

Sponsors & Collaborators

• Beijing Chao Yang Hospital: lead
• Beijing Municipal Health Commission: collaborator

Study Sites (1)

Beijing Chaoyang Hospital, Capital Medical University

Beijing, China

RECRUITING

Related Publications (2)

• Jia M, Su H, Jiang K, Wang A, Guo Z, Zhu H, Zhang F, Sun X, Shi Y, Pan X, Cao Y. Incidence and predictors of in-stent restenosis following intervention for pulmonary vein stenosis due to fibrosing mediastinitis. Orphanet J Rare Dis. 2024 Oct 14;19(1):379. doi: 10.1186/s13023-024-03391-8.

  PMID: 39397011 BACKGROUND

• Varghese C, Johnson GB, Eiken PW, Edell ES, Specks U, Larson NB, Peikert T. A Retrospective Evaluation of the Treatment Effects of Rituximab in Patients with Progressive and Symptomatic Fibrosing Mediastinitis. Ann Am Thorac Soc. 2024 Nov;21(11):1533-1541. doi: 10.1513/AnnalsATS.202405-533OC.

  PMID: 39106522 BACKGROUND

MeSH Terms

Conditions

Mediastinal Fibrosis; Hypertension, Pulmonary

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases

Central Study Contacts

Juanni Gong, Doctor of Medicine

CONTACT

15801511482; juannigong2018@126.com

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: RANDOMIZED
• Masking: NONE
• Purpose: TREATMENT
• Intervention Model: PARALLEL
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: April 27, 2025
• First Posted: May 20, 2025
• Study Start: February 10, 2025
• Primary Completion (Estimated): October 31, 2027
• Study Completion (Estimated): October 31, 2027
• Last Updated: May 23, 2025

Record last verified: 2025-02

Data Sharing

• IPD Sharing: Will not share

Locations

CN (1)